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anti-GJB3 antibody :: Rabbit anti-Human Connexin 31, C241 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS625667 anti-GJB3 antibody
Unit / Price
0.2 mL  /  $550 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Connexin 31, C241 (GJB3), Polyclonal Antibody

 Also Known As   

Connexin 31, CT, C241 (Gap Junction beta-3 Protein, Connexin-31, Cx31, GJB3)

 Product Synonym Names    Anti -Connexin 31, CT, C241 (Gap Junction beta-3 Protein, Connexin-31, Cx31, GJB3)
 Product Gene Name   

anti-GJB3 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 1; NC_000001.10 (35246790..35251967). Location: 1p34
 OMIM    133200
 3D Structure    ModBase 3D Structure for O75712
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
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 Specificity    Recognizes human GJB3.
 Purity/Purification    Affinity Purified
Purified by Protein G affinity chromatography.
 Form/Format    Supplied as a liquid in PBS, 0.09% sodium azide.
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 Immunogen    KLH-conjugated synthetic peptide corresponding to a portion of amino acids within aa234-264 from the C-terminal region of human GJB3.
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 Preparation and Storage    May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
 Other Notes    Small volumes of anti-GJB3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-GJB3 antibody

   Gap junctions are conduits that allow the direct cell-to-cell passage of small cytoplasmic molecules, including ions, metabolic intermediates, and second messengers, and thereby mediate intercellular metabolic and electrical communication. Gap junction channels consist of connexin protein subunits, which are encoded by a multigene family. GJBs (gap-junction proteins or connexins) play crucial functional roles associated with these channels. Defects in GJB3 have been linked to erythrokeratodermia variabilis (EKV) is an autosomal dominant genodermatosis characterized by transient figurate red patches or hyperkeratosis. Mutations in GJB2 have also been associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness.
 Product Categories/Family for anti-GJB3 antibody    Antibodies; Abs to Connexin, Junction Proteins
 Applications Tested/Suitable for anti-GJB3 antibody   

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-GJB3 antibody    Suitable for use in ELISA, Western Blot and Immunohistochemistry.
Dilution: ELISA: 1:1000
Western Blot: 1:100-1:500
Immunohistochemistry: 1:50-1:100
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NCBI/Uniprot data below describe general gene information for GJB3. It may not necessarily be applicable to this product.
 NCBI GI #    3982600
 NCBI GeneID    2707
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 UniProt Primary Accession #    O75712 [Other Products]
 UniProt Secondary Accession #    Q2TAZ8; B2R790 [Other Products]
 UniProt Related Accession #    O75712 [Other Products]
 Molecular Weight    30,818 Da [Similar Products]
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 NCBI Official Full Name    connexin 31
 NCBI Official Synonym Full Names    gap junction protein, beta 3, 31kDa
 NCBI Official Symbol    GJB3 [Similar Products]
 NCBI Official Synonym Symbols   
EKV; CX31; DFNA2; DFNA2B; FLJ22486; MGC102938
[Similar Products]
 NCBI Protein Information    gap junction beta-3 protein; connexin 31; connexin-31; OTTHUMP00000004183; OTTHUMP00000004184
 UniProt Protein Name    Gap junction beta-3 protein
 UniProt Synonym Protein Names   
Connexin-31
 Protein Family    Gap junction beta-3 protein
 UniProt Gene Name    GJB3 [Similar Products]
 UniProt Synonym Gene Names    CX31 [Similar Products]
 UniProt Entry Name    CXB3_HUMAN
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 NCBI Summary for GJB3    This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of arrays of intercellular channels that provide a route for the diffusion of low molecular weight materials from cell to cell. Mutations in this gene can cause non-syndromic deafness or erythrokeratodermia variabilis, a skin disorder. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq]
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 UniProt Comments for GJB3    GJB3: One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell. Defects in GJB3 are a cause of erythrokeratodermia variabilis (EKV). EKV is a genodermatosis characterized by the appearance of two independent skin lesions: transient figurate erythematous patches and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases. Defects in GJB3 are the cause of deafness autosomal dominant type 2B (DFNA2B). DFNA2 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Belongs to the connexin family. Beta-type (group I) subfamily.

Protein type: Membrane protein, multi-pass; Channel, misc.; Membrane protein, integral

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: connexon complex; cytoplasm; integral to membrane; gap junction

Molecular Function: gap junction channel activity

Biological Process: skin development; sensory perception of sound; in utero embryonic development; cell communication; transmembrane transport; placenta development

Disease: Deafness, Autosomal Recessive 1a; Deafness, Autosomal Dominant 2b; Erythrokeratodermia Variabilis Et Progressiva
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 Research Articles on GJB3    1. Some GJB2, GJB3, and GJB6 mutations occurred in deaf students.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Pathways associated with anti-GJB3 antibodyDiseases associated with anti-GJB3 antibody
 Products by Pathway  Pathway Diagram
 Calcium Regulation In The Cardiac Cell Pathway antibodies  Calcium Regulation In The Cardiac Cell Pathway Diagram
 Gap Junction Assembly Pathway antibodies  Gap Junction Assembly Pathway Diagram
 Gap Junction Trafficking Pathway antibodies  Gap Junction Trafficking Pathway Diagram
 Gap Junction Trafficking And Regulation Pathway antibodies  Gap Junction Trafficking And Regulation Pathway Diagram
 Membrane Trafficking Pathway antibodies  Membrane Trafficking Pathway Diagram
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >63 publications with GJB3 and Nervous System Diseases
 Skin Diseases Antibodies  >43 publications with GJB3 and Skin Diseases
 Erythrokeratodermia Variabilis Antibodies  >28 publications with GJB3 and Erythrokeratodermia Variabilis
 Hearing Loss, Sensorineural Antibodies  >25 publications with GJB3 and Hearing Loss, Sensorineural
 Erythema Antibodies  >17 publications with GJB3 and Erythema
 Congenital Abnormalities Antibodies  >9 publications with GJB3 and Congenital Abnormalities
 Neoplasms Antibodies  >4 publications with GJB3 and Neoplasms
 Peripheral Nervous System Diseases Antibodies  >4 publications with GJB3 and Peripheral Nervous System Diseases
 Hyperkeratosis, Epidermolytic Antibodies  >4 publications with GJB3 and Hyperkeratosis, Epidermolytic
 Carcinoma Antibodies  >4 publications with GJB3 and Carcinoma
Organs/Tissues associated with anti-GJB3 antibody
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >43 publications with GJB3 and Skin
 Placenta Antibodies  >9 publications with GJB3 and Placenta
 Testis Antibodies  >7 publications with GJB3 and Testis
 Brain Antibodies  >5 publications with GJB3 and Brain
 Heart Antibodies  >3 publications with GJB3 and Heart
 Eye Antibodies  >2 publications with GJB3 and Eye
 Ovary Antibodies  >1 publications with GJB3 and Ovary
 Uterus Antibodies  >1 publications with GJB3 and Uterus
 Intestine Antibodies  >1 publications with GJB3 and Intestine
 Lung Antibodies  >1 publications with GJB3 and Lung
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